RESUMEN
BACKGROUND: The aims of the study were to assess the right ventricular (RV) functions in patients with idiopathic pulmonary arterial hypertension (IPAH) with RV longitudinal strain (RVLS) in addition to conventional parameters, as well as its correlation with severity and prognosis in IPAH. METHODS: Twenty-two IPAH patients were followed up for 1 year. ANOVA and Gabriel's pairwise comparison tests were used for comparison of RVLS with respect to WHO functional class status. Patients were divided into non-survival (group 1) and survival (group 2), and clinical and echocardiographic parameters of RV function were compared at baseline and at 6 months with t-test & Mann-Whitney test. RESULTS: At baseline, with respect to WHO functional class, mean RVLS showed no significant interclass difference (P = 0.0781). Among the other conventional echocardiographic parameters, RV E/A showed significant difference at baseline (P = 0.004), but not at 6 months (P = 0.366); whereas tricuspid annular plane systolic excursion (TAPSE) which had no significant difference initially (P = 0.174) revealed a significance level at 6 months (P = 0.029) between the two groups. Fractional area change (FAC), RV index of myocardial performance (RIMP), and right atrial (RA) area displayed significant difference neither at baseline nor at 6 months. RVLS exhibited significant difference neither at baseline (P = 0.912) nor at 6 months (P = 0.181). None of the echocardiographic parameters including RVLS showed a significant average change with change in severity of PAH both at 6 and 12 months. CONCLUSION: RVLS was not proved to be a useful parameter for early detection of RV dysfunction and prognosis in patients with IPAH in comparison with the conventional echocardiographic parameters.
RESUMEN
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of non-Hodgkin's lymphoma of the skin. Clinically, SPTCL presents as subcutaneous tumors located on the extremities or trunk, often associated with systemic symptoms like fever or fatigue. The therapeutic regimen for SPTCL is at present not standardized. We describe herein a case of a young woman who presented with intermittent fever and skin rash and was diagnosed later with SPTCL. The case is reported here for its rarity and rapidly changing unusual clinical manifestations. This case also highlights that monotherapy with systemic steroid can be a valuable treatment option for the management of SPTCL, especially in those without hemophagocytic syndrome.
